GBE1, Polyclonal Antibody

4-alpha-glucan-branching enzyme; Brancher enzyme; Glycogen-branching enzyme; GBE1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This GBE1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 527-556 amino acids from the Central region of human GBE1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-GBE1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

GBE1 is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease).

Cancer; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunofluorescence (IF)

WB~~1:1000

Western blot analysis of GBE1 Antibody (Center) in HL-60 cell line lysates (35ug/lane). GBE1 (arrow) was detected using the purified Pab;

Western blot analysis of GBE1 Antibody (Center) in mouse liver tissue lysates (35ug/lane).GBE1 (arrow) was detected using the purified Pab.

Confocal immunofluorescent analysis of GBE1 Antibody (Center) with HepG2 cell followed by Alexa Fluor? 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red). DAPI was used to stain the cell nuclear (blue).

80474

glucan (1,4-alpha-), branching enzyme 1

1,4-alpha-glucan-branching enzyme

1,4-alpha-glucan-branching enzyme

GLGB_HUMAN

The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq, Jul 2008]

GBE1: a monomeric enzyme that functions in glycogen symthesis by catalyzing the formation of alpha 1,6- glucosidic linkages.Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. It is most highly expressed in liver and muscle. Deficiency can result in glycogen storage disease IV (Andersen's disease).

Protein type: Carbohydrate Metabolism - starch and sucrose; Transferase; EC 2.4.1.18

Chromosomal Location of Human Ortholog: 3p12.3

Cellular Component: cytosol

Molecular Function: cation binding; 1,4-alpha-glucan branching enzyme activity; hydrolase activity, hydrolyzing O-glycosyl compounds

Biological Process: glycogen metabolic process; generation of precursor metabolites and energy; glycogen biosynthetic process; carbohydrate metabolic process; glucose metabolic process; pathogenesis

Disease: Polyglucosan Body Disease, Adult Form; Glycogen Storage Disease Iv

Konstantinidou, A.E., et al. Placenta 29(4):378-381(2008)
Massa, R., et al. Muscle Nerve 37(4):530-536(2008)
Bruno, C., et al. Acta Myol 26(1):75-78(2007)

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